Multiple progressive occlusions of intracranial arteries

OBJECTIVES: We present a case of multiple progressive occlusions of intracranial arteries, a variety of Taveras' syndrome, without smoke spirals, which may be confused with other multifocal disorders. PATIENT: A 30 year old woman was admitted to hospital with a clinical picture of fluctuating paresia of her left limbs, blurred vision and urgency of micturition which partially recovered. On examination there was paresia of the left arm, generalized increased reflexes and facial asymmetry. RESULTS: On MRI there were areas of marked hyperintensity at T2. Some of these took up gadolinium at T1 and had a serpiginous pattern, compatible with vasculopathy. On angiography, stenosis and filling defect were seen in the left carotid artery. The anterior cerebral and left Sylvian arteries were filled by the vertebrobasilar system via the posterior communicating artery and an anomalous trigeminal artery. CONCLUSIONS: The clinical picture of multiple progressive occlusions of intracranial arteries is a variety of moya-moya disease, without the typical smoke spirals, which may lead to confusion with clinical pictures of arteritis and demyelinating disorders if angiography is not done, since this is essential for diagnosis.